Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Feb 1;33(1):40-48.
doi: 10.1097/MOP.0000000000000988. Epub 2020 Dec 29.

New approaches to risk stratification for Wilms tumor

Affiliations
Review

New approaches to risk stratification for Wilms tumor

Marie V Nelson et al. Curr Opin Pediatr. .

Abstract

Purpose of review: The treatment of Wilms tumor is one of the great achievements in the field of oncology. One of the key success factors has been improved risk stratification, enabling augmentation or reduction of therapy depending on a patient's risk of relapse. This article highlights the evolution of clinical and biological prognostic markers that have been applied in the treatment of Wilms tumor.

Recent findings: Historically, tumor stage and histology were the sole determinants of Wilms tumor treatment. Recent clinical trials conducted by the Children's Oncology Group (COG) and the International Society of Pediatric Oncology (SIOP) Renal Tumor Study Group have expanded the menu of prognostic factors to include histologic and volumetric response to therapy and tumor-specific loss of heterozygosity (LOH) at chromosomes 1p and 16q. Augmentation of therapy has been able to overcome the adverse risk factors. An emerging prognostic marker is chromosome 1q gain, will be incorporated into future clinical trials.

Summary: The application of new clinical and biological prognostic factors has created unprecedented ability to tailor therapy for Wilms tumor, accompanied with improved outcomes. Current and future trials will continue to enhance precision medicine for Wilms tumor.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Evolution of clinical, pathological and molecular prognostic factors over successive studies conducted by the National Wilms Tumor Study Group /Children's Oncology Group and the International Society of Pediatric Oncology (SIOP) Renal Tumor Study Group. The prognostic factors depicted in red are new prognostic factors that were added for a given generation of study.

References

    1. Chu A, Heck J, Ribeiro KB, Brennan P, Boffeta P, Buffler P, Hung RJ. “Wilms’ tumour: a systematic review of risk factors and meta-analysis.” Paediatric and Perinatal Epidemiology. 2010; 24:449–469. - PubMed
    1. Howlader N, Noone AM, Krapcho M, et al. (eds). SEER Cancer Statistics Review, 1975–2014, National Cancer Institute. Bethesda, MD, https://seer.cancer.gov/csr/1975_2014/, based on November 2016 SEER data submission, posted to the SEER web site, April 2017.
    1. Scott RH, Stiller CA, Walker L, et al. “Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour.” J Med Genet. 2006; 43 (9):705–15. - PMC - PubMed
    1. Mahamdallie S, Yost S, Poyastro-Pearson E, Holt E, Zachariou A, Seal S, et al. Identification of new Wilms tumour predisposition genes: an exome sequencing study. The Lancet Child & Adolescent Health. 2019;3(5):322–31. - PMC - PubMed
    1. Irtan S, Ehrlich P, and Pritchard-Jones K. “Wilms tumor: ‘State-of-the-art’ update, 2016.” Seminars in Pediatric Surgery. 2016; 25(5): 250–256. - PubMed

Publication types